Bleeding Disorders

Overview

Bleeding disorders affect the way the body controls blood clotting. If your blood does not clot enough, you may experience problems with bleeding too much after an injury or surgery. Blood that clots too much can lead to other medical conditions, such as thrombotic disorders. This health topic focuses on bleeding disorders that are caused by problems with clotting factors, including hemophilia and von Willebrand disease.

Bleeding disorders can be inherited, meaning a person is born with the disorder, or acquired, meaning the disorder develops during a person’s life. Acquired bleeding disorders are more common than inherited bleeding disorders. You may develop a bleeding disorder if something, such as a disease or a medicine, causes your body to stop making blood clotting factors or causes the blood clotting factors to stop working correctly. Problems with your blood vessels can also lead to bleeding.

Related Terms: Coagulation disorders, clotting factor disorders

Signs and Symptoms

Symptoms may vary with the type of bleeding disorder and its cause. Signs and symptoms may be obvious soon after a child is born or may not be noticed until adulthood. Bleeding disorders can have symptoms that range from mild, such as sporadic nosebleeds, to severe, such as hemorrhagic stroke. People who have a mild bleeding disorder may not experience any symptoms until they are injured or have a medical procedure.

Signs and symptoms of bleeding disorders may include:

• Large bruises and bruising often
• Petechiae (tiny purple, red, or brown spots caused by bleeding under the skin) 
• Excessive bleeding that does not stop with pressure and may start spontaneously, such as with nosebleeds, or after a cut, dental procedure, or surgery 
• Heavy menstrual bleeding, which includes having periods that often last longer than seven days or require changing sanitary pads or tampons more than every hour 
• Blood in urine or stool 
• Heavy bleeding after giving birth 
• Umbilical stump bleeding that lasts longer than what is typical for newborns — about 1 to 2 weeks after the umbilical cord is cut — or that does not stop
• Redness, swelling, stiffness, or pain from bleeding into muscles or joints, which is particularly common with inherited hemophilia 

Related Health Effects

Heart disease is more difficult to manage if a person has a bleeding disorder. In addition, women with bleeding disorders have unique needs, especially during pregnancy.

Causes and Risk Factors

When a blood vessel is damaged, the body begins a process called coagulation, which thickens the blood into a mass called a clot to stop the bleeding. If blood does not clot normally, a person may bleed too much after an injury or medical procedure.

Clotting factors, also called coagulation factors, are proteins in the blood that work with small cells called platelets to form blood clots. Any problem that affects the function or number of clotting factors or platelets can lead to a bleeding disorder.

Genes, or other causes such as medical conditions or medicines, can cause bleeding disorders. Sometimes, it may be impossible to know the cause. Hemophilia is usually an inherited bleeding disorder, meaning it is passed down from parents and is present at birth. However, about one-third of babies diagnosed with hemophilia have no family history of the disorder. Von Willebrand disease is almost always inherited, as parents pass the gene for the disease on to their children.

Medical conditions, procedures, or medicines can also cause bleeding disorders. Some medical conditions and medicines that lead to bleeding disorders cause the body to produce proteins, called antibodies or inhibitors, that can block clotting factors. Other medical conditions may cause the body to stop making clotting factors or to make too little of them. Certain medical devices or procedures can also affect clotting factors, because blood flow through the area can be affected.

Medical conditions that can increase the risk for acquired bleeding disorders include:

• Blood transfusions
• Bowel diseases or bowel surgery
• Cancer and malignancy
• Congenital or acquired heart diseases
• Hypothyroidism
• Immune disorders, including autoimmune diseases such as rheumatoid arthritis or lupus
• Infections
• Liver disease
• Pregnancy
• Postpartum bleeding, which can use up the body’s clotting factors too quickly
• Skin conditions
• Trauma, or severe injury, to the brain or body
• Lymphoproliferative disorders, such as certain types of leukemia

Medical treatments that raise the risk for bleeding disorders include:

• Antibiotics
• Blood thinner medicines, also called anticoagulants, which help prevent blood clots from forming inside the blood vessels
• Drugs that prevent platelets from clumping together, including clopidogrel (Plavix), ticagrelor (Brilinta), and prasugrel (Effient)
• Nonsteroidal Anti-inflammatory Drugs (NSAIDs), including common over-the-counter pain relievers like aspirin, ibuprofen (Advil, Motrin), and naproxen (Aleve)
• Selective serotonin reuptake inhibitors (SSRI) antidepressants, such as fluoxetine and sertraline, which can affect platelet function
• Steroids used to treat inflammation, like prednisone
• Interferon alpha, a medicine used to treat certain types of cancer
• Devices that increase blood flow, such as ventricular assist devices
• Surgeries that can lead to acquired von Willebrand disease, such as heart surgeries that use a heart-lung bypass machine

Other risk factors for bleeding disorders include:

• Age: Bleeding disorders can happen at any age, but newborns are more likely than adults to develop bleeding caused by vitamin K deficiency and acquired hemophilia A is more common among older adults.
• Family history: Bleeding disorders may run in families, and a person has a higher risk of having a bleeding disorder if one or both parents have the disease.
• Sex: Hemophilia is much more common in men than in women, and the risk of developing bleeding disorders such as acquired hemophilia is higher in women during and after pregnancy.

Some herbal supplements, including high-dose vitamin E, garlic, and gingko balboa, can raise the risk for bleeding disorders by interfering with blood clotting.

Prevention Guidance

Inherited bleeding disorders, such as hemophilia and von Willebrand disease, cannot be prevented. People who are planning to have children and know that they are at risk of having a child with a bleeding disorder may want to meet with a genetic counselor, who can answer questions about the risk.

If you are diagnosed with a bleeding disorder, your health care provider may discuss your risk of passing the condition onto a future child. Depending on risk factors, a provider may recommend screening the baby during pregnancy using chorionic villus sampling (CVS), which involves taking a tissue sample from the placenta to test for genetic disorders, or testing fetal cells from amniotic fluid (amniocentesis) later in pregnancy. If a person is a hemophilia carrier and is pregnant, the provider may offer CVS at 10 to 12 weeks to check for the hemophilia gene.

Because newborns have a higher risk of vitamin K deficiency bleeding, a baby will probably receive a vitamin K shot right after birth to prevent a related bleeding disorder.

Diagnosis

A healthcare provider may diagnose a bleeding disorder, and determine whether it is inherited or acquired, based on symptoms, risk factors, medical and family history, a physical exam, and diagnostic tests. Typically, providers screen for bleeding disorders only if a patient has known risk factors or before certain major surgeries.

To help diagnose a bleeding disorder, a healthcare provider may ask questions such as:

• Do you have blood in your urine or stool?
• Does your family have a history of bleeding disorders, bleeding events, or blood clots?
• Do you have a history of miscarriage or postpartum bleeding?
• Do you have symptoms of other medical conditions linked to bleeding disorders?
• How long does your menstrual period last, and how many sanitary pads or tampons do you use per day during your period?
• How often do you bruise or bleed, and how long does the bleeding last?
• What prescription or over-the-counter medicines, such as aspirin, do you take?
• Do your gums often bleed, or do you bleed a lot following dental procedures?

Physical Examination

A healthcare provider may do a physical exam to look for signs of a bleeding disorder, such as bruising or petechiae, and to check for other medical conditions that could lead to bleeding disorders or cause similar symptoms. Depending on the reason for the visit, the provider may also look for signs of bleeding-related complications by checking for swelling and measuring blood pressure, heart rate, and breathing.

Screening Tests

Screening tests can help determine if blood is clotting properly:

• A complete blood count (CBC) measures many different parts of the blood, such as red and white blood cells and platelets. If the number of platelets is low, a person may have a platelet disorder instead of a clotting factor disorder.
• A partial thromboplastin time (PTT) test, also called an activated PTT (aPTT), measures how long it takes blood to clot and can help determine whether there is a problem with a certain group of clotting factors.
• A prothrombin time (PT/INR) test also measures how long it takes blood to clot and can help identify problems with a different group of clotting factors than the PTT test.
• A fibrinogen test measures levels of fibrinogen, which is essential for forming stable clots.

Clotting Factor Tests

To determine which bleeding disorder a person has, a healthcare provider may order two other types of tests focused on specific clotting factors. Factor activity tests measure how well a specific clotting factor functions to stop bleeding, while factor antigen tests measure the amount of a specific clotting factor in the blood. Examples of these tests include:

• Specific Factor tests: These can identify deficiencies in specific proteins. Factor VIII (8) and IX (9) tests, for example, are crucial for diagnosing Hemophilia A and B, respectively.
• Von Willebrand Factor tests: These test the amount and function of Von Willebrand Factor, typically used to diagnose von Willebrand disease.

Tests for Other Medical Conditions

A healthcare provider may also perform tests to check for conditions that can cause acquired bleeding disorders. Examples of these tests include, but are not limited to:

• A liver panel looks for signs of liver damage and can help determine whether liver problems are causing the bleeding disorder.
• A lupus anticoagulant test checks for antibodies that may be a sign of an autoimmune condition affecting how blood clots.
• Procedures such as a pelvic exam, Pap test, ultrasounds, or biopsy of the lining of the uterus can help look for signs of disorders that can cause heavy menstrual bleeding.

Treatment and Management

Bleeding disorders affect many parts of the body, so a team of specialized healthcare providers may be needed for care. This often includes a hematologist, a doctor who specializes in blood disorders, and may also include a specialized nurse, physical therapist, and social worker. Treatments and specialty care can be given at hemophilia treatment centers (HTCs), which are often the best choice to support overall health and wellbeing.

Treatment for bleeding disorders will vary depending on the type of disorder and may include medicines and factor replacement therapy. Treatment may be needed every day to prevent bleeding episodes, or as needed when planning for surgery or after an accident. A person may not need treatment if their bleeding disorder causes few or no symptoms.

Medicines

The following medicines may help treat bleeding disorders:

• Antifibrinolytic agents, such as tranexamic acid, help treat bleeding after childbirth or during dental work and other procedures.
• Birth control pills can help lower heavy menstrual bleeding in Von Willebrand disease.
• Desmopressin (DDAVP), a human-made hormone, can help stop minor bleeding in hemophilia or Von Willebrand disease.
• Immunosuppressive medicines, such as prednisone, help block production of antibodies in acquired bleeding disorders.
• Monoclonal antibodies can imitate the missing factor to help blood form clots. For example, emicizumab is an antibody that bridges factors IX and X to mimic the way factor VIII works, which can help treat people with hemophilia A.
• Vitamin K supplements treat vitamin K deficiency bleeding.

Gene Therapy

Gene therapies exist for treating certain bleeding disorders. Hemgenix and Beqvez are FDA-approved, one-time injections to treat hemophilia B in adults, while Roctavian has been developed for adults with severe hemophilia A.

Factor replacement therapy

A healthcare provider may recommend factor replacement therapy when a person experiences bleeding or to prevent bleeding from occurring. Treatment with replacement therapy on a regular basis to prevent bleeding is called prophylactic treatment.

Factor replacement therapy may include different components:

• Clotting factor concentrates replace the missing clotting factor in the blood. This treatment can raise the risk of developing antibodies that block clotting factors, which can make a bleeding disorder harder to treat, though factor concentrates in higher amounts can sometimes still help.
• Fresh frozen plasma, from human blood, contains all the clotting factors and can help treat bleeding disorders that occur when multiple clotting factors are missing, such as liver disease-associated bleeding. They can also be used during massive blood component transfusion protocols in trauma and surgery.
• Bypassing agents can help blood clot when antibodies that block clotting factors are causing the bleeding disorder. This treatment can raise the risk of blood clots forming in the blood vessels.

Bleeding disorders may also be treated with factor replacement therapy. This is a type of treatment where clotting factors isolated from blood donations or made in a lab are given to replace the missing clotting factor responsible for a bleeding disorder. Hemophilia A and B are often treated in this way because they are caused by missing clotting factors. This can be done by injecting clotting factors into the blood, a process called infusing, and patients are able to do this themselves, which allows them to prevent most bleeding issues.

Living With a Bleeding Disorder

Coping and living with a bleeding disorder involves a proactive, team-based approach focused on comprehensive care, medicine adherence, and safe, active lifestyles. Key strategies include routine prophylactic (preventative) treatment, access to specialized care, exercising to protect joints, and managing the emotional impact through community support. Connecting with local groups can help a person meet other families in their area who are also affected.

Even if a person does not need to treat a bleeding disorder, a provider may recommend taking precautions before a medical procedure or during a pregnancy to prevent bleeding problems in the future. A person should also talk to their doctor about what to do in an emergency and discuss which medicines are safe to take.

If a person has a child with a bleeding disorder, they should talk with the child’s healthcare team or a bleeding disorders treatment center about ways to protect the child at home and school.

Research Information

The National Institutes of Health (NIH) is the largest public funder of biomedical research in the world. NIH invests most of its budget in medical research seeking to enhance life and to reduce illness and disability. NIH-funded research has led to breakthroughs and new treatments helping people live longer, healthier lives, and building the research foundation that drives discovery.  

NIH is funding research and clinical studies that advance our understanding of bleeding disorders and developing new treatment options, including gene therapies that could cure certain bleeding disorders.

Find NIH-funded research projects using NIH RePORTER, a searchable database of current and past research projects supported by NIH and other federal agencies.